血管性血友病因子裂解酶功能检测在血栓性血小板减少性紫癜诊断中的意义  

Significance of measurement of von Willebrand factor-cleaving protease activity in diagnosis of thrombotic thrombocytopenic purpura

作　　者：苏健[1] 白霞[1] 刘芳[1] 高维强[1] 余自强[1] 王兆钺[1] 阮长耿[1] 

机构地区：[1]苏州大学附属第一医院江苏省血液研究所,215006

出　　处：《中华检验医学杂志》2006年第5期414-416,共3页Chinese Journal of Laboratory Medicine

基　　金：国家自然科学基金资助项目(30470732);江苏省"135工程"重点学科开放课题基金资助项目(135XY0404)

摘　　要：目的建立血栓性血小板减少性紫癜(TTP)的实验诊断方法,并用于TTP患者的诊断。方法将微管透析前、后的正常人混合血浆、TTP患者血浆进行琼脂糖凝胶水平电泳与WesternBlot,分析TTP患者血浆中vWF多聚体的组成变化;以残余胶原结合力检测血管性血友病因子裂解酶(vWF-cp)的活性水平。结果正常人混合血浆的vWF-cp活性为88·79%,4例TTP患者vWF-cp活性分别为8·90%,4·72%,28·73%,10·35%。正常混合血浆透析后只存在小分子量vWF;TTP患者血浆透析前、后的多聚体分布无变化或变化不明显。结论残余胶原结合力测定结合裂解后的vWF多聚体电泳与Western Blot技术可准确分析vWF-cp活性与vWF多聚体结构组成的变化,为TTP诊断提供新的有用的实验诊断方法。Objective To establish the experimental method for diagnosis of thrombotic thrombocytopenic purpura （TTP）. Methods The plasma of the patients with TTP and the normal pooled plasma （NP）, which were prepared by either dialysis or not, were involved in the study and the gel eleetrophoresis and Western Blot were performed to investigate the structural difference of vWF multimers in the TTP patients. The yon Willebrand factor-cleaving protease （vWF-ep） activity was analyzed with the residue-collagen binding assay （R-CBA）. Results The vWF-Cp aetivity was 88.79% in NP and 8.90%, 4. 72%, 28.73% and 10. 35% in four patients with TTP respectively. In TTP, there was no significant structural difference of vWF multimers in the plasma between before and after dialysis while only low molecular weight vWF was observed in dialyzed NP. Conclusion The combination of the gel eleetrophoresis and Western Blot as well as R-CBA is helpful for the laboratory diagnosis of TTP.

关 键 词：von Willebrand病 紫癜 血栓性血小板减少性 

分 类 号：R554.6[医药卫生—血液循环系统疾病] R541.4[医药卫生—内科学]